Mokahal, Ali El

Abstract Rationale In the early post-lung transplant period, close surveillance is essential to detect allograft dysfunction, infection, or medication toxicity. This requires frequent in-person clinic visits and laboratory testing, creating substantial travel and time burdens for patients and caregivers. Remote patient monitoring (RPM), which uses devices to collect and transmit physiologic and symptom data to the care team, has the potential to reduce geographic barriers and enable earlier detection of changes in patient status. Understanding the feasibility, adherence, and clinical impact of RPM is essential before broader implementation. Methods The Chronic Lung Allograft Monitoring (CLAM) program was launched in August 2024 at our center. Participants were provided a tablet and devices (spirometer, pulse oximeter, scale, and blood pressure cuff) to submit daily physiologic and symptom data. Abnormal parameters triggered review by RPM nurses and escalation to transplant physicians according to protocol. After 12 months of monitoring, patients were transitioned to self-monitoring when clinically appropriate. Demographics, compliance, and escalation characteristics were summarized descriptively. An escalation was defined as any entry triggering an alert to the transplant team. Escalations were categorized by cause and reviewed to determine clinical impact. Results Among 105 enrolled patients, the median age was 64 (IQR 58-68) years, 66 (63%) were male. 70 (67%) remain enrolled, 22 (21%) graduated, and 13 (12%) discontinued monitoring prematurely. reasons for discontinuation included death (n = 7; 7%), patient preference (n = 3; 3%), and non-adherence (n = 3; 3%), yielding an overall dropout rate of 5.7%. Completion rates were 64% for vital sign and spirometry submissions and 91% for symptom questionnaires.There were 395 escalations, with a median of 3 per patient (IQR 1-6); 82 patients (78%) experienced at least one escalation. Causes of escalations are summarized in Figure 1. Of all escalations, 282 (71%) were managed with continued monitoring, while 113 (29%) resulted in changes in care. Seven escalations (1.8%) led to emergency department evaluation and 2 (0.5%) resulted in direct hospital admission. Among non-emergent escalations, 35 (8.8%) prompted expedited clinic visits; of the remainder, 42 (10.6%) led to diagnostic testing orders and 32 (8.0%) resulted in medication initiation or adjustment. Conclusions RPM after lung transplantation was feasible, well accepted, and frequently informed clinical decision-making. The CLAM program demonstrates high engagement and clinical utility, supporting further prospective evaluation of RPM in lung transplant care. This abstract is funded by: None

Abstract Rationale Gastroesophageal reflux disease (GERD) is a common comorbidity in non-cystic fibrosis bronchiectasis (NCFB), yet its impact on clinical outcomes remains poorly defined. We sought to determine whether GERD is associated with worse clinical outcomes in patients with NCFB using a large, real-world database. Methods We conducted a retrospective cohort study using the TriNetX platform, identifying patients with NCFB (ICD-10 J47*) with and without concurrent GERD (ICD-10 K21*), excluding those with cystic fibrosis (ICD-10 E84*). Propensity score matching (PSM) was performed using 1:1 nearest neighbor matching without replacement, controlling for age, sex, race/ethnicity, obesity, socioeconomic risk factors, and tobacco use. Primary outcomes included pneumonia, bronchiectasis exacerbations, intensive care unit admission, mechanical ventilation, and mortality. Statistical analyses included risk ratios and Cox proportional hazards modeling for survival outcomes. Results Out of 421,710 patients with NCFB in the dataset from January 2000 to September 2025, 186,874 (44%) had a diagnosis of GERD. After propensity score matching, 179,753 patients with NCFB+GERD and 179,753 patients with NCFB-only were included (mean age 74.0±13.1 years, 58.8% female, 67.1% White). Baseline characteristics were well-balanced after matching. Median follow-up was approximately 2.5 years for both cohorts (898 vs 889 days; similar means and IQR), ensuring balanced observation time. Compared to NCFB-only, NCFB+GERD was associated with increased risks for mortality (risk ratio 1.04, 95% CI 1.02-1.05; p < 0.001), pneumonia (risk ratio 1.06, 95% CI 1.05-1.08; p < 0.001), bronchiectasis exacerbations (risk ratio 1.03, 95% CI 1.01-1.06; p = 0.006), and mechanical ventilation (risk ratio 1.14, 95% CI 1.10-1.18; p < 0.001). Intensive care unit admission rates were comparable between groups (risk ratio 1.00; p = 0.65). Conclusions In this large propensity score-matched analysis, concurrent GERD in NCFB is independently associated with increased mortality, pneumonia, bronchiectasis exacerbations, and mechanical ventilation requirements. These findings suggest GERD may represent a modifiable risk factor warranting aggressive management in NCFB patients. Study limitations include retrospective design, reliance on ICD-10 coding accuracy, and potential unmeasured confounders. Our results support systematic screening and treatment of GERD in NCFB, but whether targeted GERD management can modify the natural history of NCFB future investigation. This abstract is funded by: None

Abstract Introduction: We present a case of constrictive bronchiolitis with paraneoplastic autoimmune multi-organ syndrome (PAMS) treated with lung transplantation. To our knowledge, this is the first reported case of PAMS with constrictive bronchiolitis managed with this approach. Presentation: A 45-year-old woman presented with 3 months of dyspnea. PFTs showed severe airflow obstruction with air trapping and no immediate bronchodilator response. [FEV1 0.94L (z: -3.9), FVC 2.98L (z: -0.515), TLC 4.89L (z: 1.0), RV 1.70L (z: 2.4) and DLCO 22 ml/min/mmhg (z: 0.58)] CT chest with expiratory views showed air trapping in the left upper lobe and ground glass opacities, consistent with constrictive bronchiolitis. 6 months prior, she was diagnosed with abdominal grade 1 follicular lymphoma, treated with Bendamustine and Rituximab. One month into treatment, she developed a lichenoid rash involving the oro-gential mucosa and erythematous papular skin eruptions. Her eruptions and constrictive bronchiolitis were consistent with PAMS with pulmonary involvement. The rash improved with oral steroids and topical therapies, but her pulmonary symptoms did not. The likelihood of progression was low, so lymphoma was not a contraindication for lung transplantation. To ensure complete metabolic remission, she was further treated with R-CHOP and maintained on Rituximab. During transplant evaluation, FEV1 was 30% with NYHA class II, Karnofsky score 60 and she required 4L oxygen. She grew mycobacterium abscessus subspecies abscessus on 3 different sets of induced sputum. Secondary to radiographic findings consistent with NTM-PD (bronchial wall thickening and mucoid impaction) and pending lung transplant, she was treated with omadacycline, tedizolid, amikacin and clofazimine until sputum culture negativity (3-months). She underwent lung transplantation and received pre-operative Mycophenolate and Methylprednisolone without induction immunosuppression. Post-operatively she was started on Tacrolimus, Mycophenolate Mofetil and Prednisone. M. Abscessus therapy continued for 2 months post-transplant. At 6 months post-transplant, she is well, and PFTs are improving without evidence of organ rejection, lymphoma recurrence, or M. abscessus infection. Discussion: Pulmonary involvement in PAMS is associated with high mortality and is not reversed by treatment of the neoplasm, due to non-reversible destruction of small airways. The only disease modifying therapy is lung transplant. Her lymphoma recurrence risk was very low after 2 years of remission following initial treatment, and as her lymphoma was not associated with immunosuppression, post-transplant immunosuppression likely does not increase risk of recurrence. Additionally, while mycobacterium abscessus poses significant risks, intensive treatment in the peri-transplant period can help allow for safe transplantation.

Abstract Introduction:IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory condition marked by lymphoplasmacytic infiltrates that tend to affect various organs, leading to diverse clinical manifestations. While commonly linked to conditions like pancreatitis and sialadenitis, it can also impact the lungs, causing pulmonary masses, consolidations, or pneumonia. Key histopathological features include IgG4-positive plasma cells and storiform fibrosis, although these may not always be present. Case Presentation: We present a case of an 82-year-old male with a past medical history significant for pulmonary cryptococcal infection and a previous COVID-19 infection, who presented to our pulmonology clinic with fatigue for two months followed by an upper respiratory infection that did not respond to several rounds of antibiotics. A chest CT scan revealed bilateral consolidative airspace opacities and lymphadenopathy. Subsequent bronchoscopy with transbronchial biopsy and bronchoalveolar lavage (BAL) suggested a possible cryptococcal infection; however, his symptoms did not improve after a course of fluconazole. A follow-up PET-CT scan showed increased metabolic activity in the bilateral pulmonary consolidations, thoracic adenopathy, a possible right supraclavicular lymph node, and bilateral inguinal lymph nodes. He underwent a repeat bronchoscopy with EBUS-guided biopsy of the FDG-avid lymphadenopathy, and pathology results indicated granulomatous inflammation. He was evaluated by ophthalmology for uveitis and signs of retinal detachment on brain MRI, as well as nephrology for a progressive decline in kidney function. A renal biopsy showed numerous plasma cells staining for IgG and IgG4, along with storiform interstitial nephritis, consistent with IgG4-related interstitial nephritis. His autoimmune evaluation revealed elevated levels of ANA, double-stranded DNA, RNP antibodies, and various IgG subclasses, including IgG1, IgG3, and IgG4. Considering the biopsy results and the reduced suspicion of an infectious process, he was initiated on immunosuppression treatment with prednisone and rituximab for the IgG4-related disease which resulted in significant improvement in both the patient's symptoms and kidney function. Discussion:In conclusion, this case emphasizes the diagnostic challenges of IgG4-related pulmonary disease, largely due to the nonspecific nature of the respiratory manifestations. In our case, the diagnosis remained unclear despite the patient undergoing both transbronchial and EBUS-guided lymph node biopsies. This highlights the importance of maintaining a high clinical suspicion for this underdiagnosed condition as early treatment can lead to notable recovery.

Abstract Introduction Abemaciclib is a selective CDK4/6 inhibitor approved for metastatic breast cancer. We report the clinical course of Abemaciclib-induced pneumonitis, emphasizing the need for vigilant monitioring for interstitial lung disease (ILD) in patients receiving this therapy. Case description An 85-year-old woman with a history of HR-positive, HER2-negative breast cancer, Atrial fibrillation, and stage 3 chronic kidney disease presented with cough and weakness and a temperature of 100.1° F. She was hypoxic, requiring 4 liters of oxygen, she was in atrial fibrillation with a heart rate of 114, and blood pressure was 98/46. Laboratory testing showed an NT-Pro BNP: 5768, leukocytes: 2900 with 420 lymphocytes and an acutely elevated creatinine of 1.83 mg/dL (basline 1.3-1.4 mg/dL). Her CRP was 245 mg/L. Chest X-Ray revealed diffuse interstitial opacities. She was treated for pneumonia with cefepime and azithromycin, volume resuscitation and subsequent diuresis. However, her hypoxia continued to worsen. A chest CTA excluded pulmonary embolism but revealed progression of diffuse ground-glass and reticular opacities with a basilar apical gradient and enlarged mediastinal and hilar lymph nodes. An echocardiogram showed a mildly enlarged right ventricle and elevated right ventricular systolic pressure of 60. Subsequent infectious and autoimmune workups were inconclusive, bronchoscopy evaluation was deferred due worsening respiratory status. Given the concern for drug induced pneumonitis she was started on a steroid taper with 2 mgs/kg of IV Methylprednisolone. Over two weeks, her condition improved, and she was discharged without supplemental oxygen. Discussion Pneumonitis is a rare adverse effect of abemaciclib likely driven from CDK4/6 inhibitors. In a safety analysis of the MONARCH 2&3 trials, the incidence of pneumonitis was 2-5%, compared to 0.4-0.6% in the placebo arms. In these trials, two and one patients died of pneumonitis respectively. Our patient received abemaciclib for 2 years and 10 months, during which her follow up PET CT scan few months after treatment initiation demonstrated a new ground-glass and reticular opacities that continued to worsen on subsequent surveillance PET CT scans with significant progression noted on admission CT. In the absence of other contributing factors, our patient was diagnosed with Grade 3 lung toxicity, and abemaciclib was discontinued. The progression of her imaging before admission highlights the need for close monitoring for interstitial lung disease in patients receiving abemaciclib, to determine if dose reduction or treatment adjustment is warranted.